BackgroundProgressive Hemifacial Atrophy (PHA) is an acquired, typically unilateral, facial distortion with unknown etiology. The true incidence of this disorder has not been reported, but it is often regarded as a subtype of localized scleroderma. Historically, a debate existed whether PHA is a form of linear scleroderma, called morphea en coup de sabre (ECDS), or whether these conditions are inherently different processes or appear on a spectrum (; Adv Exp Med Biol 455:101–4, 1999; J Eur Acad Dermatol Venereol 19:403–4, 2005). Currently, it is generally accepted that both diseases exist on a spectrum of localized scleroderma and often coexist.The pathogenesis of PHA has not been delineated, but trauma, autoimmunity, infection, and autonomic dysregulation have all been suggested. The majority of patients have initial manifestations in the first two decades of life; however, late presentations in 6th and 7th decades are also described [J Am Acad Dermatol 56:257–63, 2007; J Postgrad Med 51:135–6, 2005; Neurology 61:674–6, 2003]. The typical course of PHA is slow progression over 2-20 years and eventually reaching quiescence.Systemic associations of PHA are protean, but neurological manifestations of seizures and headaches are common [J Am Acad Dermatol 56:257–63, 2007; Neurology 48:1013–8, 1997; Semin Arthritis Rheum 43:335–47, 2013]. As in many rare diseases, standard guidelines for imaging, treatment, and follow-up are not defined.MethodsThis review is based on a literature search using PubMed including original articles, reviews, cases and clinical guidelines. The search terms were “idiopathic hemifacial atrophy”, “Parry-Romberg syndrome”, “Romberg’s syndrome”, “progressive hemifacial atrophy”, “progressive facial hemiatrophy”, “juvenile localized scleroderma”, “linear scleroderma”, and “morphea en coup de sabre”. The goal of this review is to summarize clinical findings, theories of pathogenesis, diagnosis, clinical course, and proposed treatments of progressive hemifacial atrophy using a detailed review of literature.Inclusion- and exclusion criteriaReview articles were used to identify primary papers of interest while retrospective cohort studies, case series, case reports, and treatment analyses in the English language literature or available translations of international literature were included.

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Postoperative pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterized by PG at surgical incisions. It is often misdiagnosed as wound infection, and pathergy may complicate wound debridement. From September 1, 2013, through November 30, 2013, a literature search was conducted of articles published from January 1, 1978, through December 31, 2012. We referenced PubMed, MEDLINE, and Mayo Clinic Libraries using the keywords pyoderma gangrenosum, postoperative pyoderma gangrenosum, postsurgical pyoderma gangrenosum, superficial granulomatous pyoderma, pathergic pyoderma, and pyoderma gangrenosum associated with surgery, incision, breast, and wound dehiscence. In addition, all titles from PubMed with the words pyoderma gangrenosum were reviewed manually for postoperative cases. Of 136 patients, 90 (66%) did not have associated systemic diseases. If a comorbidity was present, hematologic disorders were most common. In addition, 29% (28) of women had predisposing disease vs 53% (19) of men. Women had more frequent breast involvement (P<.001); chest involvement was more common in men (P=.005). Girls and women aged 13 to 64 years had more frequent breast involvement (P=.01). Sites were distributed equally for men regardless of age (P=.40). Antibiotic drug therapy was begun and debridement occurred in 90% (110 of 122 treated patients) and 73% (90 of 123 available patients), respectively. Postoperative PG has less association with systemic disease than its nonpostoperative counterpart. Antibiotic drug therapy is routinely initiated. Affected sites are often debrided, causing additional wound breakdown. Early diagnosis may prevent complications.

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